Spitz nevi (epithelioid and spindle-cell nevi) are a special group of melanocytic neoplasms with a specific clinical, dermatoscopic and histological picture. There are typical and atypical Spitz nevus. The typical Spitz nevus is more common in pediatric practice and has a benign course. Of particular interest are atypical forms that combine the characteristics of a typical Spitz nevus and melanoma. The article presents an analysis of the clinical picture, dermatoscopic, immunohistochemical, histological and genetic characteristics in various forms of Spitz nevus. The existing classification according to the metastasis risk degree is presented. The last recommendations on the tactics of managing patients with this nosology are discussed with examples of original observations. © 2021, Pediatria Ltd.. All rights reserved.